Mucoepidermoid Carcinoma

INTRODUCTION Mucoepidermoid carcinoma, also known as adenosquamous carcinoma, is a tumor of low- and high-grade malignancy. Low-grade tumors can appear at any age and grow slowly. The highgrade lesions are more infiltrative and metastasize aggressively to the regional lymph nodes and to distant sites. They typically arise from the major and minor salivary gland epithelium and ductal elements, where they account for 10% to 30% of all primary carcinomas. However, they can also arise within the lacrimal and accessory lacrimal gland tissues, lung, esophagus, and upper respiratory tract. It can also rarely present primarily in the skin or arise from the lacrimal sac.

CLINICAL PRESENTATION On the eyelids, mucoepidermoid carcinomas involve the lacrimal and accessory glands and present as a subconjunctival or less commonly a subcutaneous nodule. The lid margin may be red, thickened, and indurated with telangiectasias and foci of ulceration. The overlying skin can be involved through direct extension and may occasionally be ulcerated. Pain, when present, is more often associated with the high-grade type. If the lacrimal sac is involved presentation is with a medial canthal mass simulating dacryocystitis. In some cases the tumor may arise within the skin from sweat gland epithelium. In such cases a well-circumscribed subepithelial cystic mass is palpable without fixation to the overlying epithelium. Tumor can extend backward into the orbit. Enlarged preauricular lymph nodes may be palpable.

Preauricular Lymph Node Palpation

(Courtesy of Seymour Brownstein, M.D. Source: (Courtesy of Charles S. Soparkar, M.D.)

From JW Robinson, S. Brownstein, et al.

Conjunctival mucoepidermoid carcinoma in a patient with ocular cicatricial pemphigoid and a review of the literature. Surv Ophthalmol 2006; 51:513-519, with permission of Elsevier.)

(Courtesy of Seymour Brownstein, M.D. Source: (Courtesy of Charles S. Soparkar, M.D.)

From JW Robinson, S. Brownstein, et al.

Conjunctival mucoepidermoid carcinoma in a patient with ocular cicatricial pemphigoid and a review of the literature. Surv Ophthalmol 2006; 51:513-519, with permission of Elsevier.)

HISTOPATHOLOGY This tumor is composed of a mixture of squamous epithelial cells and mucus-producing goblet cells forming lobules. Goblet cells predominate in well-differentiated tumors and squamous epithelium in poorly-differentiated neoplasms. Mucicarmine stain can be used to highlight the goblet cells, as shown on the right.

Goblet Cell Carcinoma

DIFFERENTIAL DIAGNOSIS The differential diagnosis should include chalazion, squamous cell carcinoma, sebaceous cell carcinoma, mucinous cystadenoma, metastatic tumors, and dacryocystitis.

TREATMENT Treatment is by complete surgical excision, preferably with Mohs' microsurgery or under frozen section control. Mucoepidermoid carcinoma can have a variety of clinical outcomes but the overall prognosis appears to be related to the tumor grade. For high-grade tumors the five-year survival rate is 25% to 30%, but for low-grade tumors the survival rate climbs to 80% to 95%. Sentinel node biopsy may be useful in cases of high-grade lesions.

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